TTP — RACP Adult Medicine MCQ

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ModerateHaematologyTTPRACP Adult Medicine

A 42-year-old woman presents with fever, confusion, thrombocytopaenia (platelets 25 × 10⁹/L), microangiopathic haemolytic anaemia (MAHA - schistocytes, raised LDH, low haptoglobin), and AKI. Her ADAMTS13 activity is <5%. What is the most appropriate urgent treatment?

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Correct answer: E — Plasma exchange (plasmapheresis)

The pentad of fever, MAHA, thrombocytopaenia, renal impairment, and neurological dysfunction with ADAMTS13 <10% is diagnostic of thrombotic thrombocytopaenic purpura (TTP). Plasma exchange is the mainstay of treatment and should be commenced urgently (mortality >90% without treatment). Corticosteroids and caplacizumab (anti-vWF nanobody) are adjunctive. Platelet transfusion is CONTRAINDICATED as it may worsen thrombotic microangiopathy. Rituximab is used for relapsing/refractory TTP.

Reference: eTG – 2025 – Haematology; BSH – 2023 – TTP Guidelines